The Brugada pattern is associated with a genetic disorder characterized by ST-segment elevation in the right precordial leads on electrocardiogram (EKG) in the absence of structural heart disease. Patients with the Brugada pattern have an increased risk for ventricular tachyarrhythmia and sudden cardiac death. Loss-of-function mutations in the SCN5A gene which encodes the alpha subunit of the cardiac sodium channel have been associated with Brugada syndrome (BrS). We report a case of a patient who was found to have a spontaneous type 1 Brugada pattern on a routine EKG done prior to travel. He underwent electrophysiological testing (EPS) which provoked ventricular tachycardia and underwent implantable cardioverter defibrillator (ICD) placement. His family history revealed a history of sudden cardiac death, abnormal EKG, syncope, dilated cardiomyopathy, and BrS. Genetic testing revealed a variant of uncertain significance (VUS) in the SCN5A gene in the proband and six of his relatives. The SCN5A VUS in this clinical context and segregation with the disease in his family supports its reclassification to pathogenic.

UNASSIGNED: Patients with progressive chronic kidney disease (CKD) are at higher risk of infections and complications from cardiac implantable electronic devices (CIED). In patients with a primary or secondary prophylactic indication, implantable cardiac defibrillators (ICD) can prevent sudden cardiac deaths (SCD). We retrospectively compared transvenous-ICD (TV-ICD) and intermuscularly implanted subcutaneous-ICD (S-ICD) associated infections and complication rates together with hospitalizations in recipients with stage 4 kidney disease.
UNASSIGNED: We retrospectively analyzed 70 patients from six German centers with stage 4 CKD who received either a prophylactic TV-ICD with a single right ventricular lead, 49 patients, or a S-ICD, 21 patients. Follow-Ups (FU) were performed bi-annually.
UNASSIGNED: The TV-ICD patients were significantly older. This group had more patients with a history of atrial arrhythmias and more were prescribed anti-arrhythmic medication compared with the S-ICD group. There were no significant differences for other baseline characteristics. The median and interquartile range of FU durations were 55.2 (57.6-69.3) months. During FU, patients with a TV-ICD system experienced significantly more device associated infections (n = 8, 16.3% vs. n = 0; p < 0.05), device-associated complications (n = 13, 26.5% vs. n = 1, 4.8%; p < 0.05) and device associated hospitalizations (n = 10, 20.4% vs. n = 1, 4.8%; p < 0.05).
UNASSIGNED: In this long-term FU of patients with stage 4 CKD and an indication for a prophylactic ICD, the S-ICD was associated with significantly fewer device associated infections, complications and hospitalizations compared with TV-ICDs.

Yamaguchi syndrome or apical hypertrophic cardiomyopathy is a rare subtype of non-obstructive hypertrophic cardiomyopathy that is defined as the focused hypertrophy of the left ventricular apex. It is typically seen in Asian populations. Herein, we present a rare case of Yamaguchi syndrome seen in a Hispanic female.

Implantable cardioverter defibrillators (ICD) have been recommended as an effective therapy in treating sudden cardiac deaths. This study evaluates the safety and efficacies of ICDs in detecting arrhythmias. Different ICDs, such as the transvenous cardioverter defibrillator (TV-ICD) and the subcutaneous implantable cardioverter defibrillator (S-ICD), are used. This systematic review identified Embase, PubMed, Medical Literature Analysis and Retrieval System Online (MEDLINE), and Web of Science as the primary electronic databases for research. Supplementation of the available articles for the review was done using Google Scholar. The population, exposure, control, outcome, and studies (PECOS) criteria were used in this study. The quality of the included studies was assessed using the Critical Appraisal Skills Program (CASP) standard checklist. Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines were used in this systematic review. Two researchers conducted the extraction of data. A pre-designed Excel worksheet (Microsoft, Redmond, Washington) was used in the recording of extracted data. Eight studies were identified for use in this systematic review. Safety of the ICDs was observed with the minimum number of reported inappropriate shocks. Studies conducted identified that women had a lower number of incidences when a long detection setting by sex was conducted. Strategic programming of ICDs was noted as effective in lowering the levels of mortality. Studies claimed that the reduction of inappropriate shocks were important in the reduction of myocardial damage, which resulted in the mortality rate among the patients decreasing. Having high cutoff rates and long intervals for detection in ICD programming was noted to help in reducing ICD therapy intervention among patients. Differences among the male and female populations were inconsequential in the efficacy and safety of ICDs. Their effectiveness in sensitivity, pacing success, and defibrillation success were high and very significant. ICDs were safe in their use in the detection of arrhythmias.

Cardiac sarcoidosis (CS) is a rare multisystem disorder characterized by granulomatous infiltration of the myocardium, which can lead to significant morbidity and mortality. Its clinical manifestations range from asymptomatic conduction abnormalities to severe heart failure (HF) and sudden cardiac death. This comprehensive review aims to provide an overview of the diagnosis, clinical features, and current medical management strategies for CS. Additionally, the role of implantable cardioverter-defibrillators (ICDs) and the potential use of positron emission tomography in guiding management decisions are explored. A comprehensive understanding of the medical management of CS is essential for improving patient outcomes and guiding future research endeavors.

Introduction Heart failure with reduced ejection fraction (HFrEF) patients are prone to developing ventricular arrhythmias. In the PARADIGM-HF trial, sacubitril-valsartan (SV) showed a reduction in the composite endpoint of death and HF hospitalization in HFrEF patients; subgroup analysis of this trial revealed a reduction in both sudden death and deaths from worsening HF. The mechanism by which SV may affect the incidence of ventricular arrhythmias is currently under debate, and the literature provides conflicting results. The aim of our study was to evaluate the potential antiarrhythmic effect of this drug in patients with HFrEF carrying an implantable cardiac defibrillator (ICD) or a cardiac resynchronization therapy with a defibrillator (CRT-D). Methods This was a single-center, observational and retrospective study. Inclusion criteria were implantation of an ICD or CRT-D device between 2009 and 2019, age ≥18 years, left ventricle ejection fraction (LVEF) ≤40%, New York Heart Association (NYHA) functional class ≥II, and treatment with an angiotensin-converting enzyme inhibitor or an angiotensin receptor blocker for at least 12 months, followed by replacement with SV. Exclusion criteria were NYHA class IV, frequent alterations in chronic medication for HFrEF, and implantation of an ICD or CRT-D after the introduction of SV. The primary outcome was the occurrence of ventricular arrhythmias in the form of appropriate device shocks, ventricular fibrillation, or ventricular tachycardia. The comparisons were performed between two periods of time (12 months before and 12 months after SV) in the same group of patients. Results Fifty-four patients met the inclusion criteria. The mean age was 69.5 ± 1.65 years, and 74.1% of patients were male. The number of patients experiencing appropriate shocks was significantly lower after SV initiation (2% vs. 18%; p=0.016). The percentage of VT (13 vs. 20%; p=0.549) and VF episodes (4% vs. 13% for VF; p=0.289) were also lower, but these differences were not statistically significant. There were no significant differences in the value of NT-proBNP (1128 vs. 775 pg/mL; p=0.858), LVEF (28.4 vs. 29.6%; p=0.315), and left ventricular end-diastolic diameter (65.0 vs. 66.0 mm; p=0.5492). Conclusion SV seems to reduce the risk of arrhythmic events requiring appropriate shock therapy.

Left ventricular non-compaction (LVNC), a kind of cardiomyopathy, is characterized by excessive and prominent trabeculations in the mature left ventricle (LV). LVNC has been defined as the heart\'s developmental failure to fully form the compact myocardium during the latter stages of cardiac development. Clinical features vary from asymptomatic to symptomatic individuals with gradual loss of heart function, heart failure, thromboembolic events, arrhythmias, and sudden cardiac death are all possible outcomes. We describe a case of a 39-year-old Caucasian female who presented with a sudden cardiac arrest that was later attributed to LVNC. To the best of our knowledge, only a few occurrences are found in the literature where female patients with LVNC were presented with sudden cardiac arrest.

A 51-year-old patient was admitted with chest pain and broad complex ventricular tachycardia. He received three consecutive direct cardioversion (DC) shocks and was commenced on amiodarone infusion via a central venous catheter or central line (CVC). He responded to treatment and normal sinus rhythm (NSR) was achieved. He had elevated troponin I and underwent coronary angiogram which initially was thought to be responsible for his ventricular tachycardia. Coronary angiogram (CAG) showed unobstructed coronary arteries. He was recently diagnosed with pheochromocytoma and was commenced on Phenoxybenzamine 10 mg two months back. He developed ventricular tachycardia (VT) again the next day that did not respond to four consecutive direct cardioversion shocks (DC) and antiarrhythmic medications. He was intubated and ventilated to terminate his VT and was transferred to the intensive care unit (ICU). He remained intubated for 48 hours and he remained in NSR, after which he was extubated. He was commenced on bisoprolol and was later stepped down to the coronary care unit (CCU). Cardiac magnetic resonance imaging (CMR) showed left ventricular non-compaction (LVNC) or possibly myocarditis in view of patient\'s known history of pheochromocytoma. He was discussed with surgical team at another hospital for surgical resection of the adrenal tumor and had a few further runs of VT while he was waiting to be transferred. The patient finally underwent surgical resection of the tumor and was booked for implantable cardioverter defibrillator (ICD) in view of his VT. This was an interesting case of treatment-resistant VT driven by pheochromocytoma and LVNC, and it is important to be familiar with the fact that conventional therapies may fail in these patients and may require intubation and ventilation to terminate VT storms.

For this research, we have considered a case of a man aged 60 years who developed painless fluid accumulation in the implantable cardiac defibrillator (ICD) pocket site. The cardiovascular implantable electronic device initially appeared to be infected, but it was eventually determined that the cause was an allergic reaction, and a novel solution was implemented. For patients with nickel allergies, treatment typically includes avoiding nickel or replacing with gold-plated devices with new leads. Because of the subclavian vein thrombosis, the gold-plated generator was not replaced. ICD generators were covered with antibiotic-coated envelopes while waiting for the replacement. Hypersensitivity to cardiac devices was effectively treated with this technique. By routinely using the envelope, the very rare incidence of device hypersensitivity could further be reduced.

High strength magnetic and electric fields used in magnetic resonance imaging (MRI) render images with unmatched soft tissue contrast. These imaging attributes have made MRI an increasingly preferred diagnostic tool in many medical conditions. Initially there was substantial concern regarding the safety of performing these imaging studies in patients with cardiac implantable electronic devices (CIEDs), which have the potential to be affected by the intense electric and magnetic fields of the MRI. More recently, there has been increasing evidence that MRI can be performed safely in patients with devices that have not been specifically labelled by regulatory agencies for use in an MRI environment (MRI nonconditional devices), which has allowed the Centers for Medicare and Medicaid Services (CMS) to start providing reimbursement for MRIs of patients with MRI nonconditional devices. For CMS to reimburse scans, a rigorous protocol must be followed, which recognizes that there are still potential adverse effects that can be mitigated by appropriate procedures. In this review we will survey the initial experiences and efforts to understand the magnitude of risk for device malfunction and harm, as well as current efforts to minimize the potential risks of MRI effects on devices and leads (heating, device movement, lead dislodgement, and device malfunction, the latter including inhibition of pacing and generation of arrhythmias).